Cerebrospinal fluid neurofilament light in suspected sporadic Creutzfeldt-Jakob disease

Publication date: Available online 9 October 2018Source: Journal of Clinical NeuroscienceAuthor(s): Eirini Kanata, Ewa Golanska, Anna Villar-Piqué, Aikaterini Karsanidou, Dimitra Dafou, Konstantinos Xanthopoulos, Matthias Schmitz, Isidro Ferrer, André Karch, Beata Sikorska, Pawel P. Liberski, Theodoros Sklaviadis, Inga Zerr, Franc LlorensAbstractSporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of human prion disease. It is invariably fatal and displays a short clinical disease stage. The key event in sCJD is the propagation of a beta-sheet rich conformer of the physiological PrPC protein, known as PrPSc. Neuropathological disease characteristics include gliosis, neuronal loss and spongiform degeneration; disease clinical manifestations refer to mental and visual disabilities, cognitive impairment, gait or limb ataxia, myoclonus and mutism. Definite sCJD diagnosis requires post-mortem brain material histopathological examination. However, highly certain pre-mortem differential diagnosis is desired to exclude other treatable disorders and to reduce disease transmission risks. Detection and/or quantification of cerebrospinal fluid (CSF) biomarkers reflecting neuronal damage and PrPC misfolding in the diseased brain significantly enhance pre-mortem diagnosis. Previously established and newly identified biomarkers are used towards this direction. Increased CSF Neurofilament light chain (NFL) concentrations have been reported in several neurological disorders, in...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research